How to recognise lymphoedema

Definition

Lymphoedema is a chronic swelling resulting from failure of lymphatic drainage. Two main types of lymphoedema are recognised:

a) Primary lymphoedema

This term describes lymphoedema where there is an intrinsic genetic abnormality of the lymphatic system. It may be inherited.

b) Secondary lymphoedema

In secondary lymphoedema, where lymphatic failure is a result of damage to an otherwise normal lymphatic system, e.g. due to surgery, radiotherapy, infection (such as cellulitis) or trauma.

However, strictly speaking, there is a lymphatic component to all types of chronic oedema. Oedema exists in the tissues whenever capillary filtration exceeds lymphatic drainage. For example, in chronic venous hypertension, capillary filtration is increased and lymphatic drainage also increases to meet the demand until the flow exceeds the maximum capacity of the lymphatics, when oedema will develop. Persistent high lymphatic flow in this situation leads to damage of the lymphatic system which then exacerbates the oedema.

In many clinical situations, the aetiology of chronic swelling is complex with a number of factors affecting capillary filtration as well as those resulting from lymphatic damage. For example, a patient may be immobile as a result of arthritis leading to a dependency-type oedema (due to impaired pumping of blood through the veins and lymph through the lymphatics by extrinsic skeletal muscle). Varicose veins may coexist. Following joint replacement surgery a degree of lymphatic damage can occur. There may be postoperative deep vein thrombosis and the patient may be on medication which can predispose to oedema development (see below).

Given the common occurrence of this type of clinical picture, the term “chronic oedema” has been coined to act as an “umbrella” description of persistent oedema of greater than 3 months duration, regardless of cause. (Moffatt et al (2003))

Nevertheless, for an individual patient it is helpful to understand the aetiology of the different types of chronic oedema as far as possible so that appropriate treatment can be determined.

The most common causes of chronic oedema are listed in Table 1.

Clinical features of chronic oedema

Lymphoedema

Lymphoedema can affect any part of the body but most commonly involves the legs. When it initially develops, the swelling is usually soft and easy to “pit” (Fig 1). However, over time, the subcutaneous tissues become firmer due to the deposition of fat and fibrosis and the swelling is less easy to “pit”.

Figure 1 Pitting oedema of the abdominal wall.

Stemmer’s sign describes the inability to pick up a fold of skin at the base of the second toe and is usually positive in lymphoedema of the legs when it has been present for some time (Figs 2a + b).

Figure 2a – Positive Stemmer’s sign right foot.

Figure 2b – Negative Stemmer’s sign left foot.

In chronic lymphoedema, skin changes such as hyperkeratosis, lymphangiectasia and papillomatosis are seen as the condition progresses. The skin becomes thickened and there can be deep skin folds and creases around the joints (Figs 3 + 4).

The distinction between primary and secondary lymphoedema is usually made on the basis of the pattern of onset and clinical history.

There are a number of types of primary lymphoedema, some of which are inherited. Some are present from birth (e.g. Milroy lymphoedema) whereas some develop around puberty (e.g. lymphoedema distichiasis) and others may develop later on in life. There is usually no identifiable cause of damage to the lymphatic system but occasionally overt lymphoedema can be precipitated by a relatively minor trauma, e.g. a sprained ankle may lead to persistent swelling in a patient with an underlying lymphatic dysplasia (Figs 5a + b).

Figure 5a – Primary lymphoedema of the foot with toe swelling and increased creasing of the toes, especially the second toe.

Figure 5b – Mild primary lymphoedema of the foot / lower leg.

In secondary lymphoedema, there should be a clear cause of lymphatic damage such as surgery, radiotherapy, infection e.g. cellulitis, trauma or lymphadenopathy due to malignancy (Fig 6).

Figure 6 – Lymphoedema of the arm following treatment for breast cancer.

There is usually evidence of chronic venous hypertension, e.g. as a result of post-thrombotic syndrome or severe varicose veins. Venous dilatation, e.g. submalleolar venous flare, telangiectasia, varicose veins, skin changes such as pigmentation especially around the gaiter area, venous eczema and lipodermatosclerosis may be present. Venous ulcers may develop and in severe cases the limb may develop an inverted champagne bottle appearance.

This typically presents as soft oedema in an immobile patient, beginning in the feet and working up the legs. It is usually bilateral but can be unilateral in patients with a localised weakness, e.g. a hemiparesis, in which case it may also affect the arm on that side. Patients usually have a history of long-standing medical problems such as neurological conditions, e.g. multiple sclerosis, chronic respiratory problems e.g. COPD or chronic musculoskeletal problems such as arthritis.

Typically, the patient spends a lot of the day sitting in a chair and may even sleep in a chair at night, albeit often a reclining chair.

The oedema is usually very soft and “pits” easily when it presents, but over time can become firmer and limb shape distortion can develop together with skin breakdown and even ulceration. Chronic “leaky legs” may be a problem.

Obesity

Chronic oedema is extremely common in severely obese patients. Sometimes it has the features of lymphoedema with typical skin changes as described above, whereas in others there may be a clear pattern of venous disease. In severely obese patients immobility may contribute to the picture.

The typical pattern is to develop oedema in the lower part of the legs initially, but it may extend to include the whole leg. Some patients with extreme obesity have an abdominal “apron” and oedema may develop in the dependent part of this. Skin changes typical of chronic lymphoedema may be seen in the oedematous apron (see above).

There is a separate condition, lipoedema, which can sometimes be confused with chronic oedema in obesity (Fig 9).

Figure 9 – Lipoedema of the legs with ‘shouldering’ at the ankles.

Lipoedema is a type of lipodystrophy in which there is an increased deposition of fat in the lower part of the body, typically from the hips down to the ankles, with marked shouldering of the fat at the level of the ankles, with the feet normally being unaffected. It seems to develop in teenage years and occurs exclusively in women. There is sometimes a family history. The clinical picture is one of non-pitting symmetrical enlargement of the legs with the feet being normal and Stemmer’s sign being negative. The legs may be tender and bruise easily. A typical feature of the history is that attempts to lose weight usually result in weight loss in the upper part of the body but not from the legs.

If lipoedema has been present for some years, superadded pitting oedema can develop, particularly in the feet and ankles. This condition is then known as lipolymphoedema.

Oedema due to heart failure

Peripheral oedema is commonly recognised in patients with acute heart failure but is also present in those with chronic heart failure (Fig 10). In people with reduced mobility, the clinical features can be similar to those of a dependency-type oedema.

However, there are usually other features suggestive of heart disease, e.g. ischaemic heart disease, cor pulmonale, valvular disease or cardiomyopathy. Typically, the oedema affects both legs, although it may not be symmetrical and it often extends on to the sacral region. Other clinical features of heart failure such as bilateral basal crackles, raised jugular pressure, etc. may be present.

Oedema of advanced cancer

Although localised oedema may be a feature of a number of advanced cancers, e.g. breast cancer and head and neck cancer, a particularly common pattern develops in patients with advanced pelvic disease.

The oedema can be extremely extensive going from the toes up to the abdomen and possibly involving the genitalia. It is usually quite soft and easy to pit with the overlying skin being stretched. However, in some cases it can become quite firm. Leakage of lymph (lymphorrhoea) can occur.

The aetiology is often multifactorial and may include: pelvic lymphadenopathy, extrinsic venous compression including inferior vena caval obstruction, deep vein thrombosis, ascites, hypoalbuminaemia and immobility. Severe oedema with hypoalbuminaemia can be particularly difficult to manage.